Original Contribution

Transient Monocular Vision Loss on Awakening: A Benign

Amaurotic Phenomenon
Marc A. Bouffard, MD, Wayne T. Cornblath, MD, Joseph F. Rizzo III, MD,
Michael S. Lee, MD, Lindsey B. DeLott, MD, Eric R. Eggenberger, DO,
Nurhan Torun, MD, FRCS(C)
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Background: Transient monocular vision loss (TMVL) is an and were uniformly negative. In 14 patients for whom clear
alarming symptom owing to potentially serious etiologies follow-up data could be obtained, no medically or visually
such as thromboembolism or giant cell arteritis. Our significant sequelae of this syndrome were found, and 50%
objective is to describe the phenomenon of TMVL present experienced resolution of symptoms.
on awakening, which may represent a distinct and benign Conclusions: Evaluation was uniformly negative when
entity. patients described waking with isolated vision loss in 1
Methods: We performed a retrospective observational case eye with subsequent resolution, usually in less than
series of 29 patients who experienced TMVL on awakening. 15 minutes. The natural history seems benign with
Patients who described monocular dimming or blackout of symptoms frequently remitting spontaneously. This visual
vision were included, and those with blurred vision, concur- phenomenon may represent an autoregulatory failure
rent eye pain, and binocular vision loss were excluded. resulting in a supply/demand mismatch during low-light
Descriptive statistics were used to summarize the study conditions.
population.
Results: Of the 29 patients we studied, 90% (n = 26) were Journal of Neuro-Ophthalmology 2017;37:122–125
female and 48% had crowded discs (cup-to-disc ratio #0.2). doi: 10.1097/WNO.0000000000000451
The mean age was 45.4 years, although women were sig- © 2016 by North American Neuro-Ophthalmology Society
nificantly younger than men (mean ages 43.4 and 62.7
years, respectively, P = 0.017). Brain magnetic resonance

T
imaging and vascular imaging (magnetic resonance angiog- ransient monocular vision loss (TMVL) may indicate
raphy, computed tomographic angiography, or carotid Dopp- serious underlying disease. Diminished blood flow to
ler) were performed in 69% and 55% of cases, respectively,
the orbit, retina, or choroid is usually due to atherosclerotic
or inflammatory vascular conditions. Increases in intraocular
Department of Neurology (MAB), Beth Israel Deaconess Medical
Center and Harvard Medical School, Boston, Massachusetts;
pressure or intracranial pressure from any etiology can also
Department of Ophthalmology and Visual Sciences (WTC, LBD), transiently impair the function of the optic nerve. Retinal
Kellogg Eye Center, University of Michigan Medical Center, Ann vasospasm and transient vision loss due to optic disc drusen
Arbor, Michigan; Department of Ophthalmology (JFR), Massachu-
setts Eye and Ear Infirmary and Harvard Medical School, Boston,
represent benign causes of TMVL (1,2). We report a distinct
Massachusetts; Department of Ophthalmology (MSL), University of phenomenon of isolated TMVL on awakening not previ-
Minnesota, Minneapolis, Minnesota; Departments of Neurology and ously described in the literature. This phenomenon is dis-
Ophthalmology (ERE), Michigan State University, East Lansing,
Michigan; and Department of Surgery (NT), Division of Ophthal-
tinguished from other syndromes of TMVL by the presence
mology, Beth Israel Deaconess Medical Center and Harvard Medical of isolated and stereotypic visual symptoms present on wak-
School, Boston, Massachusetts. ing with rapid resolution, normal neuro-ophthalmologic
One group’s series (N.T., M.A.B.) was presented at the 41st Annual examination, and negative work-up for potential causes.
Meeting of the North American Neuro-Ophthalmology Society,
February 21–26, 2015, San Diego, CA.
The authors report no conflicts of interest. METHODS
Supplemental digital content is available for this article. Direct URL
citations appear in the printed text and are provided in the full text Approval was obtained from the institutional review boards
and PDF versions of this article on the journal’s Web site (www. at all participating medical institutions where necessary, to
jneuro-ophthalmology.com).
review the charts of 52 patients seen for transient, recurrent
Address correspondence to Marc A. Bouffard, MD, Department of
Neurology, Kirstein Building, 4th Floor, Beth Israel Deaconess Medical visual changes on awakening. All patients were evaluated by
Center, Boston, MA 02215; E-mail: marc_bouffard@meei.harvard.edu neuro-ophthalmologists at 1 of 5 institutions: the

122 Bouffard et al: J Neuro-Ophthalmol 2017; 37: 122-125

Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
 Original Contribution

Longwood Eye Clinic at the Beth Israel Deaconess Medical reported onset of symptoms after turning the lights off.
Center, the Kellogg Eye Center at the University of Queries as to whether or not the episodes resolved on light
Michigan, the Massachusetts Eye and Ear Infirmary, exposure only became part of the history taking for this
Michigan State University, and the University of Minne- group of patients after one of the patients in one examiner’s
sota. Patients were included if there was well documented, series (NT) volunteered this observation. As such,
isolated, monocular dimming, or blackout of the entire field a minimum of 28% of patients (8/29) were illumination-
of vision immediately present on waking and with complete dependent and of the 11 patients in which we have clear
eventual resolution. Patients with monocular blurred vision data, 73% resolved with light exposure. This question was
(which would suggest ocular surface disease or difficulty not asked by the other examiners although 1 patient eval-
focusing), binocular symptoms, and concurrent eye pain or uated by one of the authors (MSL) did describe
redness (suggestive of anterior segment pathology) were illumination-dependent symptoms. Three patients reported
excluded. We did not exclude patients with preexisting this phenomenon only on the side on which they had slept;
neurologic or ophthalmologic conditions. Descriptive sta- however, sleeping habits were not recorded for other
tistics were used to tabulate data on our patients and patients.
a 2-tailed unpaired t test was applied to determine whether Comorbidities were infrequent. Systemic hypertension
there was a significant difference in age between men and was seen in 5 patients (17%), hyperlipidemia in 4 (14%),
women. and diabetes in 1 (3%). A history of migraine was present in
3 patients (10%), although there was no temporal associ-
ation between migraine headache and the episodes of
RESULTS TMVL. History of idiopathic intracranial hypertension
Our clinical findings are summarized in Supplemental without papilledema, systemic lupus erythematosus, Ray-
Digital Content 1 (see Table E1, http://links.lww.com/ naud phenomenon, and sarcoidosis were present in 1
WNO/A212). Twenty-nine patients (26 women and 3 patient each.
men), aged 20–67 years, met the inclusion criteria. The One patient was in the first trimester of pregnancy. One
mean age of our patients was 45.5 years with a significant patient was taking an oral contraceptive. Two patients were
difference between women (mean age 43.3 years, SD 12.8) on an estrogen-based hormonal replacement regimen.
and men (mean age 62.6 years, SD 4.0), P = 0.017. All patients examined had normal intraocular pressures
The left eye was involved in 9 patients (31%), the right and normal iridocorneal angles. Crowded discs (as defined
eye in 13 patients (45%), and either eye in 7 (24%). by a cup-to-disc ratio of # 0.2 or examiner notation of
Duration of visual symptoms was clearly described in 27 of “crowded disc” or “obliterated cup”) were present in 48%
the 29 patients. Spells were usually of sort duration with all of patients where the information was available (13 of 27
but 3 lasting less than 15 minutes. Almost a quarter of patients); the fundus examination was otherwise normal. Of
events (22%) lasted 30 seconds or less. Episodes lasting the 4 patients who had asymmetric cup-to-disc ratios, 1
between 30 seconds and 15 minutes comprised two-thirds patient had symptoms on the side of the smaller disc. In
(67%) of our series. Three patients (11%) had spells lasting that case, the disc ipsilateral to symptoms was crowded
longer than 15 minutes, reported as 45 minutes, 1 hour, (cup:disc 0.2). The remaining 7 eyes with asymmetric cup-
and 4 hours. These longer episodes occurred in patients ping had cup:disc ratios of 0.3–0.7.
who had only a single spell. Given that this is a retrospective review, the diagnostic
Spells were typically infrequent; 6 patients (21%) had evaluation was performed at the discretion of the clinician
only 1 spell and 23 (79%) reported fewer than 10 at the and work-up was not uniform because there are no
time of initial neuro-ophthalmologic evaluation. However, guidelines in the literature for this unique presentation.
in the remaining 21%, spells were frequent and often Brain magnetic resonance imaging was performed in 19
occurred several times per week for months to years before patients (69%). Vascular imaging of the head and/or neck
our initial evaluation. Of the 23 patients who had more was performed in 15 patients (55%, a combination of
than 1 spell, frequency data could be obtained in 19. Of magnetic resonance angiography, computed tomographic
those 19 patients, 1 (5%) had daily spells, 4 (21%) had 1 or angiography, and carotid Doppler), whereas transthoracic
more spell per week but not daily, 8 (42%) had 1 or more echocardiography was performed in 9 (31%). Elevated
per month but not weekly, and 6 (32%) had 1 or more per sedimentation rate (ESR) and C-reactive protein (CRP)
year but not on a monthly basis. The average time from were performed in 6 patients (21%). Work-up did not
symptom onset to initial evaluation was difficult to quantify reveal relevant findings in any patients.
but ranged from several weeks to 15 years. These data were Fifteen of the 29 patients were seen only once in clinic.
obtained from 21 of the 29 patients. Follow-up data were available in the remaining 14
Data regarding the luminance-dependence of symptoms patients. Seven patients (50%) reported resolution of
are incomplete. At least 8 of the 29 patients experienced episodes over a mean follow-up period of 32.4 months
rapid resolution on light exposure. Two patients also (median 24.8 months). Reduction in frequency was seen

Bouffard et al: J Neuro-Ophthalmol 2017; 37: 122-125 123

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 Original Contribution

in 5 of the 14 (36%) over a mean follow-up duration of The monocular nature of symptoms may be caused by
13.2 months (median 5.6 months). Persistent symptoms a combination of anatomic factors and dynamic changes in
at their original frequency were seen in 2 patients (14%) vascular tone of the retrobulbar vessels. Many disorders of
with a mean follow-up duration of 2.4 months. None of altered vascular tone can affect the vascular tree in an
the patients experienced stroke or persistent visual loss asymmetric manner; reversible cerebral vasoconstriction
during follow-up. syndrome (RCVS) and migraine with aura are well-known
examples. Menstruation, presumably due to hormonal
fluctuations, may trigger headache in many migraineurs,
and RCVS predominantly affects women. Although there
DISCUSSION are scant data on the impact of crowded discs on ocular
Transient-isolated monocular vision loss often carries impor- blood flow directly, patients with optic disc drusen may
tant diagnostic implications; many cases are caused by present with TMVL (1). Crowded discs were present in
potentially life or sight-threatening etiologies. However, almost half of our patients and may play a role in the
dimming or blackout of vision present on awakening with pathogenesis of this disorder.
subsequent resolution and without other deficits seems to In conclusion, TMVL on awakening is a novel phe-
comprise a distinct and previously undescribed neuro- nomenon with important diagnostic implications. Recog-
ophthalmologic entity. In most respects, our cohort of nizing this as a benign entity will impact clinical
patients was relatively homogenous: a high proportion were management and help avoid costly testing and unnecessary
women, most had only a few episodes with a short duration anxiety. The work-up in our patients was uniformly
of symptoms, and no patient reported other symptoms that negative and no modifiable risk factors were discovered.
would be consistent with transient neurological events. All None of our patients experienced any adverse sequelae.
had a normal anterior segment examination. Most important
is the apparently benign nature of our phenomenon;
laboratory testing, as well as cardiovascular, and neuro- STATEMENT OF AUTHORSHIP
Category 1: a. Conception and design: M. A. Bouffard, W. Cornblath,
imaging studies were uniformly noncontributory. The J. F. Rizzo, M. S. Lee, L. B. DeLott, E. Eggenberger, and N. Torun;
natural history seems to be generally self-limited and benign. b. Acquisition of data: W. Cornblath, J. F. Rizzo, M. S. Lee, L. B. DeLott,
The mechanism of TMVL on awakening may reflect an E. Eggenberger, and N. Torun; c. Analysis and interpretation of data:
M. A. Bouffard, W. Cornblath, J. F. Rizzo, M. S. Lee, L. B. DeLott, E.
autoregulatory failure of the retrobulbar or retinal vascula- Eggenberger, and N. Torun. Category 2: a. Drafting the manuscript: M.
ture, resulting in a supply-demand mismatch. Absolute and A. Bouffard; b. Revising it for intellectual content: M. A. Bouffard, W.
relative changes in serum estrogen and progesterone levels Cornblath, J. F. Rizzo, M. S. Lee, L. B. DeLott, E. Eggenberger, and N.
Torun. Category 3: a. Final approval of the completed manuscript: M.
alter the resistive properties of the retrobulbar vasculature, A. Bouffard, W. Cornblath, J. F. Rizzo, M. S. Lee, L. B. DeLott, E.
which might account, to some extent, for the female Eggenberger, and N. Torun.
predominance in our series and the higher age in men
(3–14). Altering the resistive indices of these vessels may
disrupt normal neurovascular autoregulation, resulting in
insufficient retinal flow as metabolic demand increases
under conditions of low light (15–18). ACKNOWLEDGMENTS
Retinal metabolic demand is higher in darkness than in
light, likely accounting for the preponderance of subjects M. A. Bouffard was responsible for data collection, analysis,
reporting the phenomenon in dimly lit rooms. In darkness, manuscript preparation, and manuscript revision. N. Torun,
rods and cones generate “dark current,” the continuous depo- W. Cornblath, J. F. Rizzo, M. S. Lee, L. B. DeLott, and E.
larization of photoreceptors, which causes neurotransmitter Eggenberger were responsible for data collection and
release (15). Ocular blood flow, retinal glucose and oxygen manuscript revision. All authors had full access to data.
consumption are all higher in darkness and reduced on expo- These data are neither under review nor have they been
sure to constant light, consistent with neurovascular coupling published elsewhere.
(16–18). The prompt resolution of symptoms on light expo-
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